Investigation for the pathogenic mechanism of the sensorineural hearing loss of PMP gene

本研究旨在探讨PMP基因的分离及其在内耳和耳蜗功能障碍中的分子机制，以期解决腓骨肌萎缩症（Charcot-Marie-Tooth disease）感音神经性耳聋的发病机制。尽管在激光共聚焦显微镜下，抗pmp抗体在动物模型靠近外毛细胞的部分显示出强烈的免疫组织化学反应，但我们不能确定其在Corti器官中的定位和听力损失的原因。

We investigated the isolation and molecular mechanism of the PMP gene in the inner ear and cochlear dysfunction in order to solve the mechanism of the sensorineural hearing loss for Charcot-Marie-Tooth disease . Although the portion close to the outer hair cells of the animal models demonstrated intense immunohistochemical reaction by the anti-pmp antibody using confocal laser scanning microscopy, we could not identify thespecification of localization in the organ of Corti and the cause of hearing loss.